Maternal health and early outcome in pregnant woman with eisenmenger syndrome and ebstein anomaly

Abstract

Eisenmenger syndrome (or ES, Eisenmenger reaction or tardive cyanosis) is defined as the process in which a left to right shunt caused by a congenital heart defect in the fetal heart causes increased flow through the pulmonary vasculature, causing pulmonary hypertension, (1) which in turn causes increased pressures in the right side of the heart and reversal of the shunt into a right-to-left shunt. Eisenmenger syndrome is a cyanotic heart defect characterized by a long-standing intracardiac shunt (caused by "VSD": Ventricular septal defect, "PDA": Patent ductus arteriosus, or, less commonly, "ASD": Atrial septal defect) that eventually reverses to a right-to-left shunt. This syndrome is less frequent today because of medical screening with echocardiography early in life. Eisenmenger syndrome specifically refers to the combination of a cardiac shunt (systemic-to-pulmonary), significant enough to cause cyanosis and overtime pulmonary hypertension. Pregnancy should ideally be avoided in a woman with Eisenmenger syndrome, since it carries a high (approximately 50 percent) risk of sudden death for mother, frequently occurring a few days postpartum (2).However, a case of Eisenmenger syndrome and Ebstein anomaly in pregnancy where the patient's postpartum complications were successfully managed is reported.